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Erythromelalgia in Child
See also in: Cellulitis DDx
Other Resources UpToDate PubMed

Erythromelalgia in Child

See also in: Cellulitis DDx
Contributors: David O'Connell MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Erythromelalgia is a rare condition classically presenting with intermittent symptoms of severe paroxysmal burning sensation, erythema, and warmth of the skin, usually involving the extremities, lower more often than upper. It has also been reported to involve the face, ears, and genital area.

Erythromelalgia may be primary or secondary, with primary erythromelalgia (PE) further separated by some into a primary inherited form and an idiopathic group. Others consider only the inherited form as PE. This primary inherited form is due to an autosomal dominant mutation in the SCN9A gene on chromosome 2q that encodes for Nav 1.7 (voltage-gated sodium channels present in small nociceptive neurons). By contrast, secondary erythromelalgia can be associated with a multitude of underlying conditions. These include myeloproliferative disorders with thrombocythemia, in addition to vascular, connective tissue, neurological, and musculoskeletal conditions. Some drug reactions have been reported to trigger erythromelalgia, as have poisonings and intoxications.

Poisoning from ingestion of the mushroom Paralepistopsis (formerly Clitocybe) acromelalga causes a self-limited form of erythromelalgia. Onset is within 2-3 days of ingestion, and symptoms usually resolve in 1-2 weeks but may persist for months.

Incidence estimates have ranged from 0.3 to 1.3/100 000 for all forms of erythromelalgia, and these are considered low due to underdiagnosis. Some studies suggest a female predominance of up to 2-3:1, and there does not appear to be a racial / ethnic predilection.

Codes

ICD10CM:
I73.81 – Erythromelalgia

SNOMEDCT:
37151006 – Erythromelalgia

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Raynaud phenomenon – triggered by cold
  • Complex regional pain syndrome – more continuous, unilateral, and worsened by cold
  • Fabry disease – X-linked alpha-galactosidase deficiency on laboratory testing
  • Peripheral neuropathy – primarily sensation changes
  • Multiple sclerosis – intermittent dysesthesia may be a symptom
  • Cellulitis – localized and not chronically intermittent
  • Angioedema – urticarial swelling, lacks burning sensation
  • Vasculitis – purpuric presentation
  • Acral erythema – palmoplantar eruption seen with chemotherapy
  • Frostbite – episodic with history of cold exposure
  • Allergic contact dermatitis – scaling erythema with pruritus
  • Eosinophilic cellulitis – sudden urticarial plaques, pruritic to painful
  • Tinea pedis – erythema with pruritus and scaling noted

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:10/24/2022
Last Updated:11/09/2022
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Erythromelalgia in Child
See also in: Cellulitis DDx
A medical illustration showing key findings of Erythromelalgia : Erythema, Skin warm to touch, Feet, Hands
Clinical image of Erythromelalgia - imageId=2704003. Click to open in gallery.  caption: 'Deep red erythema of the distal digits.'
Deep red erythema of the distal digits.
Copyright © 2024 VisualDx®. All rights reserved.